WellSpan Home

How Cystic Fibrosis Affects Breathing and the Lungs

Topic Overview

Normal lung and respiratory function

The breathing tubes, or bronchi, leading to the air sacs (alveoli) are lined with cells that produce mucus. Normally, the cells produce a thin, runny mucus that coats the surface of the airways.

Foreign particles, such as dust and germs, constantly enter the airways and become trapped in the mucus. Tiny hairs called cilia on the surface of the breathing tubes sweep the mucus and foreign particles upward into the larger air passages and then up to the throat where they can be swallowed or coughed up.

Effects of cystic fibrosis on lung and respiratory function

Cystic fibrosis causes the mucus that coats the breathing tubes to become so thick and sticky that the cilia are unable to sweep the germs and other particles up and out of the lungs. The trapped bacteria lead to frequent, serious infections and permanent lung damage.

In response to infections, the body's immune system sends white blood cells to the lungs to attempt to destroy the infection. White blood cells release chemicals that kill both bacteria and surrounding normal cells. After attacking the bacteria, the white blood cells die, adding to the thickness of the mucus and destruction of the airways.

In the upper respiratory tract, thick, sticky mucus may also clog the nasal passages and sinuses. Small growths, or polyps, on the inner lining of the nose may develop from repeated infection and inflammation.

Complications

Complications of cystic fibrosis usually involve some aspect of long-term impaired respiratory function. These complications may include:

  • Coughing up blood. Coughing up small amounts of blood is often a symptom of chest infection. Coughing up very large amounts of blood can be life-threatening. If you are coughing up blood, call your doctor.
  • Pneumothorax , a buildup of air between the lungs and chest wall that causes the lung to collapse. This affects adults with cystic fibrosis more often than children.
  • Enlargement of the right side of the heart . Over time, this can cause heart failure and death.
  • Increased pressure in the lungs (pulmonary hypertension). The arteries in the lungs narrow (constrict) in response to impaired oxygen flow between the lungs and blood cells. The heart must pump harder to move blood through the blood vessels, which increases pressure in the lungs.
  • Enlarged, damaged airways (bronchiectasis) which can lead to infection.

Related Information

Credits

ByHealthwise Staff
Primary Medical ReviewerJohn Pope, MD - Pediatrics
Specialist Medical ReviewerR. Steven Tharratt, MD, MPVM, FACP, FCCP - Pulmonology, Critical Care Medicine, Medical Toxicology

Current as ofMay 4, 2017


Are you sure you would like to cancel?

All information will be lost.

Yes No ×

About the provider search

This search will provide you with WellSpan Medical Group and Northern Lancaster County (Ephrata) Medical Group primary care physicians and specialists. If we don’t have a WellSpan Medical Group physician to meet your criteria, the search will expand to include community physicians who partner with WellSpan Medical Group physicians through the WellSpan Provider Network or provide care to patients on the Medical Staffs of WellSpan’s Hospitals.

×

Schedule Your Next Appointment Online with MyWellSpan

Use your MyWellSpan patient portal any time to view available appointments, and pick the date and time that best suits your schedule.

Go to MyWellSpan

New to this practice?

If you don't have a WellSpan primary care provider and would like to schedule a new patient appointment with a provider who is accepting patients, just log into your MyWellSpan account, and go to the Appointment Center section. As you progress through the scheduling process, you will be able to see the offices that are accepting new patients in relation to your zip code. If you are not enrolled in MyWellSpan, go to https://my.wellspan.org, call 1-866-638-1842 or speak with a member of the staff at a participating facility to sign up. New patient scheduling not available at all practices/programs.

Already a patient at this practice?

If you already have a relationship with a WellSpan practice, simply log into your account, and go to the Appointment Center section. As you progress through the scheduling process, you will be able to schedule an appointment with any provider or practice that already counts you as a patient. Online scheduling varies by practice/program.

×